Undifferentiated embryonal sarcoma of the liver is so rare that only small series have been published regarding treatment. However, use of aggressive chemotherapy regimens seems to have improved the overall survival (OS). The generally accepted approach is to resect the primary tumor mass in the liver when possible. Neoadjuvant chemotherapy can be effective in decreasing an unresectable primary tumor mass, resulting in resectability.[1,2,3,4] The OS of these children appears...
Added text to provide specific examples of nonrhabdomyosarcomatous soft tissue sarcomas in the connective tissue, peripheral nervous system, smooth muscle, and vascular tissue.
Treatment of Newly Diagnosed Childhood Soft Tissue Sarcoma
Added text to state that in a study of 519 patients older than 10 years with a diagnosis of desmoid-type fibromatosis, 39 (7.5%) were found to have familial adenomatous polyposis (FAP); the patients with FAP and desmoid tumors were younger, more often male, and had more abdominal wall or mesenteric tumors than patients with desmoid tumors without FAP. Also revised text to state that a family history of colon cancer or the presence of congenital hyperplasia of the retinal pigment epithelium or location of the desmoid tumor in the abdomen or abdominal wall should prompt referral to a genetic counselor (cited Nieuwenhuis et al., Rossato et al., and Baker et al. as references 15, 16, and 17, respectively).
Added text to state that wide excision with negative margins or Mohs or modified Mohs surgery will prevent most tumors from recurring (cited Meguerditchian et al. as reference 36).
Added text to state that in retrospective reviews, adjuvant radiation therapy after incomplete excision may have decreased the likelihood of recurrence (cited Dagan et al. and Sun et al. as references 37 and 38, respectively).
Added McArthur et al. and Rutkowski et al. as references 40 and 41, respectively.
Added text to state that guidelines for workup and management of dermatofibrosarcoma protuberans have been published.
Added text to state that a single-institution retrospective review of 20 patients, including children and adults, found no difference in the probability of recurrence between patients who received chemotherapy and those who did not receive chemotherapy and suggested that radiation therapy may be useful; surgical removal of primary and recurrent tumor(s) was most effective (cited Guzzetta et al. as reference 100 and level of evidence 3iiiA).
Added text to state that liver hemangioendotheliomas may regress and then enlarge; these tumors may become malignant. Also revised text about a study of five girls with liver angiosarcoma, their presentation and progression, and author recommendations for liver ultrasound surveillance and liver transplantation.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.